Prions_Stanley B. Prusiner
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Prions_Stanley B. Prusiner
Wen-Chun Shaw
BTC 575 052 SCIENTIFIC DISCOVERY
Dr. Vankley
Stanley B. Prusiner
The Nobel Prize in Physiology
or Medicine 1997
For the discovery of "Prions a new biological principle of
infection".
Background
Stanley B. Prusiner
• Born May 28, 1942
• Chemistry, University of
Pennsylvania (1964)
• M.D., University of Pennsylvania,
School of Medicine (1968)
• Internship in Medicine, UCSF
• (1968-69)
• Residency in Neurology,
UCSF(1972-74)
• Since 1968 he has been a
professor in neurology,
biochemistry, and virology
at the University of
California in San Francisco,
and in Berkeley.
• Department of Neurology,
HSE-781, University of
California, School of
medicine, San Francisco, CA
94143-0518, USA
Prions
• Change the concept of pathology
Bacteria
Fungi
Parasites
Viruses
• Prions are simple proteins
that are much smaller than
viruses
Prions exist normally as
cellular proteins(PrPc)
PrPc can change its shape
to a harmful, diseasecausing form (PrPSc)
• PrPSc is very resistant to
high temperatures, UVirradiation and strong
degradative enzymes.
Slow virus
Scarpie virus(sheep)
Kuru(highlander in New Guinea)
CJD
Diseases
The hippocampus of mice(RML inoculation with RML scarpic
prions)
• Humans might be infected by
prions in 2 ways:
1. Acquired infection (diet and
following medical procedures
such as surgery, growth
hormone injections, corneal
transplants) i.e. infectious
agent implicated.
2. Apparent heredity(sporadic
CJD is about 1 /million per
year)
Prospect
• The mystery behind scrapie,
kuru, and BSE has finally
been unravelled.
• Other dementias may have
a similar
background(Alzheimer's
disease)
Diploma
Reference:
• http://www.nobel.se/
• http://www.ucsf.edu/neurosc/facu
lty/neuro_prusiner.html
• http://wwwmicro.msb.le.ac.uk/3035/prions.h
tml
The End